LETTER OF MEDICAL NECESSITY FOR HEREDITARY PHEOCHROMOCYTOMA/PARAGANGLIOMA GENETIC TESTING (PGLNext)

Date: Date of service/claim

To:Utilization Review Department

Insurance Company Name, Address, City, State

Re:Patient Name, DOB, ID #

ICD-10 Codes: (quick reference as suggestions:C75.5 malignant neoplasm of aortic body and other paraganglia; D35.6 benign neoplasm of aortic body and other paraganglia; D44.7 neoplasm of uncertain behavior of aortic body and other paraganglia; Z83.49 family history of other endocrine, nutritional and metabolic diseases)

This letter is in regards to my patient and your subscriber, First, Last Nameto request full coverage of medically-indicated genetic testing for hereditary paraganglioma/pheochromocytomato be performed by Ambry Genetics Corporation.

Paragangliomas (PGL) andpheochromocytomas (PCC) are endocrine tumors thought to have a hereditary component in up to 40% of cases. Those with hereditary PGL/PCC are at risk for multiple PGL/PCC, some of which have a high risk of becoming malignant. Those with hereditary conditions related to PGL/PCChave an increased lifetime risk of developing tumors and/or cancers (such as up to a 70% risk of developing renal cancer in those with von Hippel-Lindau disease, and up to a 100% risk of developing medullary thyroid cancer in those with multiple endocrine neoplasia type 2).Some of these gene mutations also increase the lifetime risk for additional cancers/tumors (like pancreatic tumors, hemangioblastomas, neurofibromas, optic gliomas, acoustic neuromas, and other neuroendocrine tumors).1,2,3

Significant aspects of my patient’s personal and/or family medical history that suggest a reasonable probability of hereditary PGL/PCC are below:

Based onthe above history, I am requesting coverage for this test (PGLNext), which analyzes 12high-risk genes associated with increased risks forPGL/PCC: FH,MAX, MEN1, NF1, RET, SDHA, SDHAF2, SDHB, SDHC, SDHD, TMEM127, VHL. Due to the history stated above, there is a reasonable probability of detecting a mutation in my patient. The clinical overlap with mutations in the above-mentioned genes makes this multi-gene test the most efficient and cost-effective way to analyze these genes.4Therefore,germline genetic testing is warranted.1,2,4

This genetic testing will help estimate my patient’s risk to develop tumors/cancer. It will also directly impact my patient’s medical management.An aggressive approach following guidelines is indicated in those that carry a mutation found by this test.Management guidelinesto reduce the morbidity and mortality associated with these tumors/cancersmay include:

  • Consideration ofCT/MRI-based screening/technologies
  • Annual biochemical screening
  • More prompt removal of tumor due to increased malignant potential (especially if a SDHB mutation is found by this test)
  • Prophylactic thyroidectomy
  • Annual ophthalmology and audiology examinations
  • Other: ______

Due to the tumor/cancer risks associated with these mutations and the interventions available, this genetic testing is medically indicated. As such, I am ordering this testing as medically necessary and affirm that my patient has provided informed consent for genetic testing.

A positive test result would confirm a genetic diagnosis and/or risk in my patient, and would ensure my patient is being managed appropriately. I am specifying Ambry Genetics Corporation because this laboratory has highly-sensitive and cost-effective testing for hereditary PGL/PCC, along with a large database of previously tested patients to ensure highly validated, accurate, and informative test interpretation.

I recommend that you support this request for coverage of diagnostic genetic testing for hereditary PGL/PCC in my patient. Genetic testing can take several weeks to complete, and the laboratory will not bill until testing is concluded. Therefore, I am requesting that the authorization be valid for 3 months.

Thank you for your time, and please don’t hesitate to contact me with any questions.

Sincerely,

Ordering Clinician Name (Signature Provided on Test Requisition Form)

(MD/DO, Clinical Nurse Specialist, Nurse-Midwives, Nurse Practitioner, Physician Assistant, Genetic Counselor*)

*Authorized clinician requirements vary by state

Test Details

CPT codes: 81403, 81404, 81405, 81406, 81408

Laboratory: Ambry Genetics Corporation (TIN 33-0892453 / NPI 1861568784), a CAP-accredited and CLIA-certified laboratory located at 7 Argonaut, Aliso Viejo, CA 92656

References:

  1. Fishbein L, et al. Inherited mutations in pheochromocytoma and paraganglioma: why all patients should be offered genetic testing. Ann Surg Oncol.2013 May;20(5):1444-50.
  2. Lenders JW,et al. Endocrine Society. Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2014 Jun;99:1915–42.
  3. Giusti F, Marini F, Brandi ML. Multiple Endocrine Neoplasia Type 1. 2005 Aug 31 [updated 2015 Feb 12]. In: Pagon RA, Adam MP, Ardinger HH, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2015.
  4. Karasek D, et al. An update on the genetics of pheochromocytoma. J Hum Hypertens. 2013 Mar;27(3):141-7.